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Classification of Systemic Vasculitis
Vasculitis may accompany a number of different diseases (secondary systemic vasculitis, SSV). Besides a paraneoplastic or infection-associated immuno-pathogenesis, direct invasion of vessels by infectious organisms or by tumor cells can lead to vasculitis. In addition, secondary vasculitis occurs in association with connective tissue disease or as drug-induced vasculitis. (
tables 3- 5).| Leukocytoclastic vasculitis including Schoenlein-Henoch's purpura | Bacterial: | streptococci, staphylococci, salmonella, yersinia, mycobateriae | Viral: | varicella-zoster, hepatitisvirus B, C, cytomegalovirus, influenza virus | Panarteritis nodosa | Bacterial: | streptococci | Viral: | hepatitisvirus A,B,C, HIV, parvovirus | Kawasaki's syndrome | Bacterial: | streptococci, samonella, yersinia, mycoplasma | Viral: | parainfluenza, rotavirus, HHV-6 |
| Neoplasia | Type of Vasculitis |
|---|---|
| Non-Hodgkin's lymphoma Hodgkin's disease Myelogenous leukaemia Adenocarcinoma Carcinoma of the bronchus |
granulomatous, lymphocytic, leukocytoclastic, or necrotizing leukocytoclastic granulomatous, leukocytoclastic, or necrotizing leukocytoclastic leukocytoclastic |
| Entity | Type of Vasculitis | |
|---|---|---|
| SLE | Systemic Lupus Erythematosus | lymphocytic proliferative vasculitis & perivasculitis with concentric fibrosis ("onion skin") |
|
Figure 10: butterfly examthema Figure 11: exanthema, dorsum Figure 12 & Figure 13: membranoproliferative glomerulitis (immuncomplex-glomerulitis) | ||
| RA | Rheumatoid Arthritis | small vessel vasculitis with intimal proliferation, little cellular infiltrate & immune deposits, early fibrinoid necrosis fibrosis of vessel wall |
|
Figure 14: vasculitis in rheumatoid arthritis Figure 15: rheumatoid granuloma with fibrinoid necrosis Figure 16 & Figure 17: rheumatoid synovitis | ||
| PSS | Primary Systemic Sclerosis (Scleroderma) | proliferating (intimal) and sclerosing vasculitis early changes: Raynaud's |
|
Figure 18 &
Figure 19: dermal atrophy, sclerosis and ulceration Figure 20 & Figure 21: dermal histology, sclerosis and degeneration of elastic membranes Figure 22: concentric sclerosis of vessels; here in kidney | ||
| SS | Sjogren's syndrome | small & medium sized vessels, variable: leukocytoclastic, mononuclear or mixed |
|
Figure 23: lymphocytic sialoadenitis, lip biopsy Figure 24: pseudolymphomatous sialoadenitis (progressing to lymphoma) | ||
| UCTD | Unclassified Connective Tissue Disease | any of above |